未经证实:在疑似重症肌无力(MG)常规进行单纤维肌电图(SFEMG)的中心,其他神经生理学研究的额外益处和肌无力模拟的频率尚未确定.我们的目的是说明神经系统和非神经性肌无力模拟的范围,以及他们的电生理评估与确认的MG的对比特征。
UNASSIGNED:我们回顾了2018年1月1日至2020年12月31日在我们中心进行的所有SFEMG研究。患者人口统计学,临床表型,记录抗体状态和最终诊断.电生理结果与临床特征和敏感性分析相关。
未经评估:共进行了528项SFEMG研究,其中213人(41%)异常。101人被诊断为MG,包括46例眼部MG和35例血清阴性疾病。与具有异常SFEMG的肌无力模拟相比,患有MG的个体具有较高的中值抖动(平均连续差异61μs与42μs,p<0.001),异常对的百分比更高(61%vs.33%,在SFEMG上p<0.001)。重复神经刺激在27.1%的MG患者中异常,并与普遍的临床表型相关(OR4.17;95%CI1.67-10.48)。13例(2%)MG患者SFEMG正常,其中10人处于临床缓解期。功能性神经疾病,颅神经麻痹,原发性眼病和肌病是常见的肌无力模拟。
未经证实:SFEMG在许多肌无力模拟中可能异常,应始终进行常规神经传导研究和肌电图检查。在常规进行SFEMG以调查可疑MG的中心,可以放弃广泛的近端重复神经刺激,而不会实质上影响诊断评估。确诊重症肌无力患者的正常SFEMG可能有助于提示临床缓解。
UNASSIGNED: In centers which routinely perform single fiber electromyography (SFEMG) for suspected myasthenia gravis (MG), the additional benefit of other neurophysiologic investigations and the frequency of myasthenia mimics has not been ascertained. We aimed to illustrate the range of neurological and non-neurological myasthenia mimics referred for evaluation, and contrast features of their electrophysiologic evaluation with confirmed MG.
UNASSIGNED: We reviewed all SFEMG studies performed at our center between 1 January 2018 and 31 December 2020. Patient demographics, clinical phenotype, antibody status and final diagnosis were recorded. Electrophysiologic findings were correlated with clinical features and sensitivity analyses performed.
UNASSIGNED: A total of 528 SFEMG studies were performed, of which 213 (41%) were abnormal. A diagnosis of MG was made in 101 individuals, including 46 with ocular MG and 35 with seronegative disease. Compared to myasthenia mimics with an abnormal SFEMG, individuals with MG had higher median jitter (mean consecutive difference 61 μs vs. 42 μs, p < 0.001) and a greater percentage of abnormal pairs (61% vs. 33%, p < 0.001) on SFEMG. Repetitive nerve stimulation was abnormal in 27.1% of people with MG and was associated with a generalized clinical phenotype (OR 4.17; 95% CI 1.67-10.48). Thirteen (2%) individuals with MG had normal SFEMG, of whom 10 were in clinical remission. Functional neurological disorders, cranial nerve palsies, primary ocular disease and myopathy were frequent myasthenia mimics.
UNASSIGNED: SFEMG can be abnormal in a number of myasthenia mimics, and routine nerve conduction studies and electromyography should always be undertaken. In centers where SFEMG is performed routinely for the investigation of suspected MG, extensive proximal repetitive nerve stimulation can be foregone without substantially affecting diagnostic evaluation. Normal SFEMG in those with confirmed myasthenia gravis may help indicate clinical remission.